Carotid lesions detected by B-mode ultrasonography in Takayasu's arteritis: "macaroni sign" as an indicator of the disease.

Twenty-three patients were studied to evaluate the clinical usefulness of high resolution B-mode ultrasonography in the detection of carotid lesions in patients with Takayasu's arteritis. In each patient the carotid arteries were examined using both B-mode ultrasonography (midfrequency of 7.5 MHz) and contrast angiography. In 19 of 23 patients, B-mode ultrasonography clearly demonstrated the characteristic circumferential arterial wall thickening of either one or both sides of the common carotid arteries as a macaroni-like, diffusely thickened intima-media complex. Conversely, contrast angiography demonstrated carotid lesions in only 13 of 23 patients. These results clearly show that B-mode ultrasonography is quite sensitive and superior in the detection of the characteristic thick intima-media complex of the common carotid artery in patients with Takayasu's arteritis, when compared with contrast angiography that is usually used for the definitive diagnosis of this disease.     

多发性大动脉炎颈动脉病变的彩超特征

目的：研究多发性大动脉炎(TA)颈动脉病变的超声特征。方法：TA患者30例。彩超检查颈总、内、外动脉，观察颈动脉病变分布，测量颈总动脉内膜中层厚度；彩色血流显像(CFI)及脉冲多普勒(PD)分析颈动脉血流变化。结果：30例患者中53支颈总动脉受累，平均内膜中层厚度2．4mm。TA颈动脉病变多累及颈总动脉近端，较少直接累及颈内外动脉，但35支颈内和31支颈外动脉内仍表现为血流异常，出现低搏动性血流。23支(23／60，38％)颈外动脉血流反向，近端颈总动脉均有严重狭窄或闭塞。4支病变颈总动脉出现壁内动脉，提示TA处于活动期。结论：彩超可发现TA颈动脉病变较多特征，对诊断TA有重要作用。     

多发性大动脉炎外周血管的超声诊断

目的 探讨多发性大动脉炎外周血管病变的超声表现。方法 采用美国GE公司生产的VIVID3彩色多普勒超声诊断仪。探头频率7.5MHz，对10例多发性大动脉炎患行二维及多普勒超声检查。结果 大动脉炎患受累血管分布为头臂动脉9例，其中锁骨下动脉9例，颈总动脉8例；腹主动脉5例，肾动脉3例，肺动脉1例，病变血管壁厚度2．0～5.0mm，管腔不同程度狭窄、闭塞及出现盗血现象。结论 超声诊断可较全面显示多发性大动脉炎形态学改变并可了解其血流动力学改变，应作为临床诊断多发性大动脉炎常规方法。     

Complete occulusion of left renal artery in pediatric-onset Takayasu's arteritis

A-16-year-old male adolescent with a 4-year history of protean clinical manifestations such as fever, abdominal pain, back pain, erythema nodosum and uveitis developed complete occlusion of left renal artery. Although he had been suspicious of having an autoimmune disease and treated with prednisolone, a definite diagnosis was not made. Finally, an angiography disclosed stenosis of abdominal aorta just beneath the origin of the renal arteries as well as complete occlusion of left renal artery. It has been reported that pediatric-onset Takayasu's arteritis sometimes shows protean clinical manifestations as in ours. Takayasu's arteritis should be considered as one of the underlaying disease, when a child develops protean manifestations suggesting an autoimmune disease.     

多发性大动脉炎中颈总动脉炎损害的彩色多普勒显像研究

目的：应用彩色多普勒诊断技术分析多发性大动脉炎中的颈总动脉炎声像图特征，从定性与定量方面提高对本病认识。方法：分析43例64条病变颈总动脉的二维及彩色多普勒声像图指标。结果：64条病变血管参数无论是在二维声像图的形态结构方面，还是在彩色血流分布及频谱形态变化方面均有显著性异常，管腔狭窄、管壁增厚。收缩期最大血流速度(Vmax)、阻力指数(RI)、收缩期加速度(Acc)及收缩期加速时间(Tacc)均增加。结论：彩色多普勒血流显像是诊断多发性大动脉炎中的颈总动脉炎损害的有效方法之一。     

Carotid artery involvement in Takayasu's arteritis: evaluation of the activity by ultrasonography.

The purpose of this study was to investigate the ultrasonographic criteria for determining the activity of carotid lesions in Takayasu's arteritis. In 10 consecutive patients, common carotid arteries with active lesions (n = 8) and inactive lesions (n = 9) were included in the study. The activity of the carotid lesions was determined on the basis of clinical and laboratory findings and computed tomographic scans. Special focus was placed on thickness and echogenicity of the involved arterial wall and outer diameter of the involved arterial segment. All 17 common carotid arteries evaluated showed segmental or diffuse circumferential thickening of the involved wall. The wall thickness was 2.5 to 5.0 mm (mean +/- SD, 3.3 +/- 0.8 mm) in active lesions and 1.1 to 2.0 mm (mean, 1.6 +/- 0.4 mm) in inactive lesions. The outer diameter of the involved segment was 7.0 to 15.0 mm (mean, 10.0 +/- 2.4 mm) in active lesions and 4.9 to 9.5 mm (mean, 6.8 +/- 1.4 mm) in inactive lesions. Active lesions showed hyperechogenicity in the full thickness of the involved wall (n = 6) or concentric triple layers with a hyperechoic middle layer (n = 2). Inactive lesions showed hyperechogenicity (n = 7) or isoechogenicity (n = 2) of the involved wall. One active lesion showed intramural arteries. Although precise measurement to tenths of a millimeter is impossible with the transducer used, prominent wall thickening with a maintained outer diameter in the common carotid artery suggests an active lesion, whereas mild wall thickening with a decreased outer diameter suggests an inactive lesion. Triple layers of a thickened wall with intramural arteries can be an ultrasonographic finding of an active lesion.     

经胸途径颈动脉重建术治疗大动脉炎脑缺血

目的：评价经胸途径颈动脉重建术治疗大动脉炎脑缺血的临床疗效。方法：总结18例患者的手术方式及疗效，本组均有颈动脉及锁骨下动脉阻塞性病变并出现严重脑缺血症状。16例升主动脉、2例主动脉弓颈动脉旁路术。结果：术后死亡3例、移植血管阻塞2例；12例术后随访平均22个月，9例症状消失或改善，移植血管累积通畅率75％（9／12）。结论：胸外途径无法重建颈动脉血流时，经胸途径颈动脉重建术是治疗大动脉炎严重脑缺血的有效手段。     

Aneurysm of the extracranial carotid artery with spontaneous echo contrast revealed by duplex sonography

A 56-year old male patient without cerebrovascular disease or risk factors presented with a painless, pulsatile right-sided cervical swelling. Ultrasonography showed a large aneurysm of the right common and internal carotid artery with homogeneous thickening of the vessel wall as well as a parietal thrombus and a dilation of the left common and internal carotid artery with markedly reduced blood flow velocities. In the enlarged lumen of the right internal carotid artery spontaneous echo contrast was apparent with slow, ineffective but orthograde blood flow motions. Ensuing diagnostic procedures revealed multiple aneurysms involving the aorta and its branches. Despite surgical removal of the aneurysm and glucocorticoid therapy, the patient died from a ruptured aneurysm of a coronary artery a few weeks later. Post-mortem examination showed panarteritis consistent with Takayasu's disease. Spontaneous echo contrast is a frequent echocardiographic finding in patients with atrial fibrillation and mitral stenosis, indicating decreased blood flow. This is associated with an increased risk of embolism. In our patient, spontaneous echo contrast indicated severely disturbed haemodynamics due to a large aneurysm of the carotid artery. In the rare case of multiple aneurysms, differential diagnosis should include dissections, infections, and connective tissue diseases. Takayasu's arteritis, however, should also be considered, which usually presents with stenoses, but may be associated with multiple aneurysms of the aorta or its branches. If the diagnostic criteria are present, immunosuppressive treatment should be initiated.     

多发性大动脉炎颈动脉受累的超声表现及活动性评估

  目的  总结多发性大动脉炎(Takayasu's arteritis, TA)颈动脉受累的声像图特点并评价超声在TA诊断及活动性评估中的作用。  方法  对58例TA患者的颈动脉进行常规超声检查, 测量受累颈总动脉管壁厚度。将TA患者分为活动期和非活动期两组, 以58名正常人作为对照, 比较各组间颈动脉管壁厚度的差异, 并使用受试者工作特征曲线分析管壁厚度对TA活动性的评估效果。  结果  58例TA患者共106条颈动脉受累, 其中76条(71.7%)管壁超声表现为弥漫性、均匀性增厚, 增厚管壁呈中等或中低回声。35条(33.0%)管壁呈通心粉征, 67条(63.2%)呈靶环征。TA活动期组管壁厚度大于非活动期组, 且均大于对照组(P均 < 0.05)。以管壁厚度来判断TA活动状态, 最佳诊断阈值为2.25 mm, 敏感性为71.4%, 特异性66.0%。按照有无管腔狭窄进一步分组, 分别确立诊断阈值后, 非狭窄组中特异性提高为81.5%, 狭窄组中敏感性提高为90.0%。  结论  靶环征是TA受累颈动脉除通心粉征外的另一超声新征象, 有助于TA的超声诊断。管壁厚度对于评估TA活动性有一定帮助。     

彩色多普勒超声对多发性大动脉炎血管病变检查的价值

目的探讨多发性大动脉炎（Takayasu＇s arteritis，TA）彩色多普勒超声检查的诊断价值。方法22例多发性大动脉炎患者经彩超检查并经临床及血管造影确诊。结果1．大动脉炎最易累及颈动脉和锁骨下动脉；2.TA超声特征：血管壁节段性环状增厚，管腔狭窄或闭塞。结论彩超可以显示动脉管壁增厚这一主要的病理特征，在TA病变的检出、测量、分型及复查等方面有重要价值。     

3例大动脉炎患者妊娠晚期剖官产术的护理

目的探讨大动脉炎患者妊娠晚期行剖宫产手术终止妊娠的术前和术后护理。方法回顾性分析3例大动脉炎患者妊娠晚期实施剖宫产手术的围手术期监护、心理护理、产科护理、活动指导、饮食护理和出院指导。结果所有患者均无先兆子痫、胎儿宫内窘迫、脑血管意外、心衰和血栓栓塞等危及患者及胎儿生命安全的并发症。结论大动脉炎患者妊娠晚期选择正确的分娩方式,加强围手术期监测和护理,能够有效的保证母婴健康,提高新生儿成活率。     

Quality of life in patients with unusual diseases: experience of nurses at the clinical research center for unusual diseases Aldo e Cele Daccò

The Information Centre for Rare Diseases (CIMR) of the Mario Negri Institute, has promoted a specific study on quality of life of patients with Takayasu's arteritis, a chronic vasculitis with unclear etiology, that affects primarily aorta and its main branches. The aim of the study is to assess the QoL of Italian Takayasu's arteritis patients referring to the CIMR, using the SF-36 Health Survey. The SF-36 questionnaire was submitted to 61 Takayasu arteritis patients who covered the inclusion criteria of the study, with a response rate of 85.25%, (6 males and 46 females). Expectedly, the overall SF-36 scores of the study population were lower compared to the general Italian population specifically with respect to General Health and Vitality scales. More extensive research would be required to explore gender differences suggested by the study.     

Thrombolytic, antiplatelet and anticoagulant therapy for thrombotic disorders]

In the antithrombotic therapy, it's important to evaluate the role of thrombus in the present clinical status of thrombotic vascular diseases such as acute arterial occlusion, arteriosclerosis obliterans or deep vein thrombosis. For this purpose, coagulofibrinolytic immunochemical molecular markers such as thrombin-antithrombin III complex and D dimer may be useful. These markers are also useful for the evaluation of therapeutic effect. For the extremely severe ischemic limb, survey and treatment of transient atrial fibrillation and possible embolic source in heart, aorta or arteries are indispensable. Moreover, coagulation abnormalities (congenital antithrombin III, protein C, protein S deficiency or acquired anti-phospholipid syndrome) or basic immunologic disorders (Takayasu's arteritis, Beh?et disease or other autoimmune diseases) should be carefully excluded or properly controlled.     

Carotid steal: report of ten cases.

The blood flow may be diverted from the external to the internal carotid artery via the carotid bulb in the absence of flow in the common carotid artery. We aimed to investigate the prevalence, hemodynamics, and clinical features of this condition. Reviewing the records of color duplex ultrasonographic examinations, we found carotid steal in 12 carotid arteries of 10 patients. In three patients flow in the ipsilateral external carotid artery was bidirectional. The steal had been demonstrated angiographically in six patients. The cause of the phenomenon was atherosclerosis, Takayasu arteritis, or trauma. Awareness of this pathway of collateral circulation may contribute to diagnostic work-up and expand management alternatives.     

Undiagnosed Takayasu's arteritis mimicking an acute aortic dissection

Takayasu's arteritis (TA) is a vasculitis involving the aorta and its branches. We report a case of undiagnosed TA that presented to the Emergency Department with a chief complaint of chest pain and signs consistent with an aortic dissection.     

Headache and Temporal Arteritis: When to Suspect and How to Manage

Temporal arteritis, also termed giant cell arteritis, is one of the vasculitides affecting large and medium sized cranial arteries, particularly of the carotid tree. Clinical manifestations may vary from the classic constellation of temporal headache in the elderly accompanied by constitutional signs, jaw claudication, and visual symptoms; therefore, a high index of clinical suspicion may be necessary to identify the disorder. Once suspected, immediate treatment is crucial while exploring any number of diagnostic tools to confirm or refute the diagnosis, since morbidity from untreated temporal arteritis can be devastating. At the same time, achieving a definitive diagnosis is paramount, as treatment can be toxic with significant morbidity of its own. Temporal artery biopsy remains the gold standard, but noninvasive diagnostic approaches are being refined. Corticosteroids remain the cornerstone of treatment, but are ineffective for, not tolerated by, or contraindicated in some individuals, necessitating the exploration of alternatives.     

Surgical treatment of Takayasu's disease

Takayasu's disease affects young females in the second and third decade of life. During the chronic phase, the aorta and its major arteries become stenotic, causing significant sequelae. Surgical treatment is possible with expectation of good results. The author advises treatment of stenotic arteries that are potentially dangerous or that adversely affect lifestyle with either percutaneous transluminal angioplasty or surgery. The author emphasizes the difference between surgical procedures for atherosclerosis versus a procedure for Takayasu's disease.     

Takayasu's arteritis diagnosed in a patient with long-standing arthralgias and arthritis

A 50-year-old woman had seronegative polyarthritis for three years, followed by vascular obstruction with classic Takayasu's arteritis. She responded symptomatically to high-dose steroid therapy and remains in long-term remission (joints and vasculature) after taper of medication. Literature review documents this relationship of arthritis to vasculitis in this and other types of vascular inflammation.     

Bayard Horton's clinicopathological description of giant cell (temporal) arteritis

The author set out to review the thought processes of Bayard Horton as he was clinicopathologically describing the first cases of temporal arteritis. The Mayo Clinic records of the original temporal arteritis patients were examined. Horton obtained the first biopsies of the temporal arteries in temporal arteritis and was the first to describe the histopathology. Horton initially thought his first two patients had actinomycosis of the temporal arteries, but later abandoned this diagnosis. He reported these two patients in 1932 as 'an undescribed form of arteritis of the temporal vessels'. He was the first to describe jaw claudication. He saw a patient with blindness and symptoms suggestive of temporal arteritis before this complication was described in the literature, but initially felt the patient had some other disease. The sedimentation rate was elevated in his first patient. He cared for the first temporal arteritis patient ever treated with cortisone.     

超声诊断多发性大动脉炎外周动脉病变的价值

目的 探讨超声诊断多发性大动脉炎外周动脉血管病变的价值。方法 采用Aeuson 128 XP 10型彩色超声诊断仪，探头频率5．0～7．5MHz，对33例多发性大动脉炎患者外周血管进行二维超声及彩色多普勒超声检查，分析病变动脉的二维及彩色多普勒声像图指标。结果 33例大动脉炎患者受累血管分布为：颈总动脉21例，锁骨下动脉14例，腹主动脉5例，肾动脉11例，股动脉1例，髂外动脉1例。病变血管壁厚度2．0～5．2mm。管腔不同程度狭窄或闭塞并出现盗血现象。结论 超声检查可全面显示多发性大动脉炎形态学改变，并可了解其血流动力学改变，是诊断大动脉炎的有力手段，可作为临床诊断多发性大动脉炎的常规方法。